Adrenocortical carcinoma, case report.

The adrenocortical carcinoma is rare and aggressive. It has a bimodal presentation, predominantly female, > 20% of cases will be diagnosed incidentally. 43-year-old male, with colic pain in the left flank, weight loss and intermittent fever. Computed tomography with a tumor on the left adrenal with liver metastases, block resection surgery was performed, pathological report of adrenocortical carcinoma with a 7 points of Weiss score and Ki67 40%. Adrenocortical carcinoma is a rare and aggressive neoplasm; the clinical presentation is variable. Systemic therapy is important even in patients with localized disease and independent of surgical approach.

El carcinoma corticoadrenal es una neoplasia rara, altamente agresiva, de distribución bimodal, con predominio en el sexo femenino, de la cual el 20% de los casos se diagnostican de manera incidental. Se presenta el caso de un varón de 43 años con dolor de tipo cólico en el flanco izquierdo, pérdida de peso y fiebre intermitente. La tomografía computarizada mostró un tumor en la glándula suprarrenal izquierda y metástasis hepática. Se realizó adrenalectomía radical en bloque, con reporte anatomopatológico de carcinoma corticoadrenal, Weiss de 7 puntos y Ki67 40%. El carcinoma corticoadrenal es una neoplasia agresiva y de presentación clínica variable. La terapia sistémica es importante incluso en pacientes con enfermedad localizada e independientemente de la cirugía.

Neoplasia oncocítica adrenocortical de potencial maligno incierto: reporte de un caso / Adrenocortical oncocytic neoplasm with uncertain malignant potential: a case report

Resumen Objetivo Describir el caso clínico de una paciente con neoplasia oncocitica adrenocortical, tratado quirúrgicamente en una clínica de Lima, Perú. Caso clínico Paciente mujer de 26 años ingresa a emergencia por dolor abdominal inespecífico. Se evidencia tumoración de 15x14x12 cm dependiente de glándula suprarrenal izquierda por lo que se decide tratamiento quirúrgico. Al análisis patológico se evidencia neoplasia oncocítica de potencial maligno incierto. Discusión Las neoplasias oncocíticas adrenocorticales son entidades poco frecuentes, con escasos reportes de casos de esta enfermedad. Para clasificarlas, se usa la escala de Weiss modificada. Obtenemos una neoplasia oncocítica de potencial maligno incierto, cuyo tratamiento incluye la cirugía de resección de tumor y observación. Conclusión Considerar a las neoplasias oncocíticas dentro del diagnóstico diferencial de incidentalomas adrenales.

Objective To describe a case report of a oncocytic adrenocortical neoplasm, treated surgically in a clinic in Lima, Peru. Case report A 26-year-old woman is admitted to the emergency due to nonspecific abdominal pain. A tumor measuring 15x14x12 cm dependent on left adrenal gland is evidenced, so surgical treatment is decided. Pathological analysis evidences oncocytic neoplasia of uncertain malignant potential. Discussion Oncocytic adrenocortical neoplasms are rare entities, with few case reports of this disease. To classify them, the modified Weiss scale is used. We obtain an oncocytic neoplasm of uncertain malignant potential, whose treatment includes surgery for tumor resection and observation. Conclusion Consider oncocytic neoplasms within the differential diagnosis of adrenal incidentalomas.

Síndromes de neoplasia endocrina múltiple: revisión radiológica / Multiple Endocrine Neoplasia Syndromes: Radiological Review

Los síndromes de neoplasia endocrina múltiple (MEN), incluyen una serie de enfermedades con alteraciones genéticas que se caracterizan por la presencia de tumores que afectan a dos o más glándulas endocrinas. Son síndromes con una herencia autosómica dominante e incluyen tres patrones: MEN 1 (síndrome de Wermer), MEN 2 (que incluye MEN 2A o síndrome de Sipple y MEN 2B o síndrome de Wagenmann-Froboese) y MEN 4. Los adenomas paratiroideos y el carcinoma medular tiroideo, son los tumores más frecuentes del MEN tipo 1 y 2 respectivamente. Esos síndromes son más comunes en pacientes jóvenes, con patología de afectación bilateral, múltiple o multifocal y, sobre todo, en pacientes con antecedentes familiares. Es necesario el trabajo en equipo de endocrinólogos, cirujanos, oncólogos y radiólogos para optimizar el tratamiento de esos pacientes.

Multiple endocrine neoplasia (MEN) encompasses a serial of familial genetically disorders in wich tumors simultaneusly occur in two or more endocrine organs. MEN síndromes are autosomal-dominant disorders categorized into three main patterns: MEN 1 (Wermer syndrome), MEN 2 (includes MEN 2A o Sipple syndrome and MEN 2B o Wagenmann-Froboese syndrome) and MEN 4. Parathyroid adenomas and medullary thyroid carcinoma are the most frecuent tumors in MEN 1 and MEN 2 respectively. These entities will be suspected in younger patients, bilateral, multiple or multifocal disease and, specially, in patients with family background. Cooperation between endocrinologist, surgeons, oncologists and radiologists is pivotal for optimizing patient treatment.

Adenoma corticoadrenal, presentándose como un incidentaloma: presentación de un caso y revisión de la literatura / Adrenocortical adenoma, presenting as an incidentaloma: case report and literature review

Resumen Objetivo: Presentamos un caso clínico con diagnóstico de incidentaloma adrenal no funcionante asintomático y analizamos las implicaciones clínicas y el abordaje realizado. Caso clínico: Se reporta el caso de un masculino de 53 años, asintomático, con hallazgo ecográfico accidental de imagen hipoecoica de contornos bien definidos en la glándula suprarrenal derecha que presentó incremento en su tamaño. Su estudio hormonal fue negativo para hiperfunción adrenal. Resultados: Se realizó suprarrenalectomía laparoscópica con técnica de 4 trocares con resección completa de la lesión. El paciente presentó buena evolución posquirúrgica. El estudio anatomopatológico concluyó el diagnóstico de adenoma corticoadrenal no funcionante. Conclusión: Ante el hallazgo de una masa adrenal mayor de 1 cm corresponde realizar una identificación hormonal y una evaluación del riesgo de malignidad en los pacientes, los cuales, junto con parámetros imagenológicos y los síntomas presentados, permitirán definir las complicaciones en el manejo y el pronóstico del paciente. El diagnóstico diferencial de los adenomas adrenales está basado en la identificación hormonal, el conocimiento radiológico y el grado de compromiso de la lesión. El abordaje laparoscópico es de elección en las lesiones pequeñas y sin sospecha de malignidad.

Objective: We present a clinical case with diagnosis of an asymptomatic nonfunctional adrenal incidentaloma, in which we discuss the clinical implications and the approach. Clinical case: Male patient, 53 years old with an accidental sonographic finding, characterized by a hypoechoic image of well-defined contours in the right adrenal gland of less than 2 cm. The hormonal test showed no adrenal hyperfunctioning. Laparoscopic adrenalectomy technique is performed with 4 trocars with complete excision of the lesion. The patient presented good postoperative evolution. Results: The pathology study showed a well-defined and benign tumor lesion of the adrenal gland, being similar to the fascicular zone and cortical hyperplasia next to it. The diagnosis is a non-functioning adenoma of the adrenal gland derived from the fascicular zone. Conclusion: Given the finding of an adrenal mass greater than 1 cm mass corresponds perform a hormonal identification and risk assessment of malignancy in patients, which with imaging parameters (echogenicity, bilateralism and the adjacent commitment) and symptoms presented allow to identify the complications in the management and prognosis of the patient. The differential diagnosis of adrenal adenomas is based on the hormonal evaluation, radiological knowledge and the commitment of the injury.

Carcinoma adrenocortical apresentando-se como massa abdominal / Adrenocortical carcinoma presenting as abdominal mass

O câncer adrenocortical (CAC) é um tumor raro com um prognóstico reservado. O CAC pode ser diagnosticado pela investigação de síndromes endócrinas, de sintomas devido ao crescimento tumoral ou de incidentaloma adrenal. A investigação hormonal demonstra na maioria dos CACs uma hipersecreção esteroidal, mas a característica dominante é uma co-secreção de cortisol e de andrógenos. A TC mostra um tumor heterogêneo e grande. Os tumores restritos à glândula adrenal têm uma evolução melhor do que tumores invasivos e metastáticos. A remoção completa do tumor é o tratamento de escolha. Nos pacientes com doença metastática ou progressiva, o tratamento com mitotano deve ser iniciado. Este relato de caso tem o intuito de demonstrar a importância do correto diagnóstico de lesões tumorais, pois têm grande influência no tratamento e prognóstico dos pacientes

Adrenocortical cancer (ACC) is a rare malign tumor with a poor prognosis. ACC can be diagnosed by the investigation of endocrine syndromes, signs and symptoms due to tumor growth or an adrenal incidentaloma. Hormonal investigations demonstrate in most ACC steroid oversecretion, but dominant characteristic being a co-secretion of cortisol and androgens. The CT shows a large heterogeneous tumor. Tumors localized to the adrenal gland have a better outcome than invasive and metastatic tumors. The complete tumor removal is the treatment of choice. In patients with metastatic or progressive disease, the treatment with mitotano must be initiated. This case has intention to demonstrate the importance of the correct diagnosis of tumors, therefore it has great influence in the treatment and prognosis of the patients

Clinics in diagnostic imaging (113).

Primary hyperaldosteronism is described in a 27-year-old Brazilian woman from an endemic area of schistosomiasis. She presented with hypokalaemia, cramps and polyuria, refractory hypertension, plasma aldosterone of 40.7 ng/dL and aldosterone/renin activity ratio higher than 100, due to an associated long-standing unsuspected aldosteronoma. Computed tomography showed a well-defined ovoid right adrenal mass, which was subsequently resected and confirmed to be an aldosteronoma. During subsequent follow-up visits, she remained asymptomatic, normokalaemic, and required no antihypertensive drugs. The differential diagnoses of refractory hypertension are discussed.

[Virilizing adrenal adenoma: a report of a case]. / Adenoma suprarrenal virilizante: comunicación de un caso.

Androgen excess can be due to different entities such as polycystic ovarian syndrome, ovarian tumors or adrenal tumors. It is presented the case of a 26 year-old woman that suffered from amenorrhea, hirsutism, voice deepening, reduction of mammary volume and 10 kg weight loss, without response to different treatments. At physical exam she had hirsutism (24 points, Ferriman-Gallaway) the clitoris had 3 cm length. Laboratory: androstenedione 29.5 ng/mL, DHEAS >1000 microg/dL, T 6.23 ng/mL, 17 OHP 4.9 ng/mL. At pelvic ultrasound the uterus and left ovary were normal, the right ovary had subcortical follicles no greater than 3 mm. The CAT scan and nuclear magnetic imaging of adrenal glands showed an oval retroperitoneal image of 7.2 x 6.5 x 8.4 cm at the right adrenal gland. Surgery was performed and the right adrenal gland excised. The histopathologic report indicated a benign cortical adenoma. The patient's postsurgical evolution was satisfactory with regression of the virilizing signs, with spontaneous return of menstrual periods. Hirsutism can be the initial sign, and even in some occasions the only one of different pathologies. The study of the hyperandrogenic patient should be integral with clinical, hormonal and imaging evaluations to be able to specify the origin of androgen production.